To report for the first time a new case of postpartum rhombencephalitis (RE) that is associated with positive anti-centromere antibody (ACA). 

Rhombencephalitis is a serious condition of the brain with multiple etiologies. Relatively uncommon, autoimmune rhombencephalitis may relapse with devastating consequences.  We report a unique case of recurrent, postpartum rhombencephalitis that is associated with positive anti-centromere antibody (ACA).  With a discussion of the case, current literature on autoimmune RE and related autoantibodies are reviewed.

The physical examination data and the results of the laboratory analyzes were collected and interpreted.

A healthy 33-year-old Caucasian patient (gravida 2, para 2) had two episodes of progressive focal neurological deficits during postpartum periods.  Patient showed signs and symptoms of right-sided dysmetria, adiadochokinesia, weakness, ataxia, and photophobia. MRI revealed rhombencephalitis involving the mesencephalon and extensive and comprehensive investigations using blood and cerebrospinal fluid (CSF) were consistently positive only for ACA. The first episode was successfully treated with empiric antimicrobial agents and steroid.  Given the negative infectious work up in prior episode and the nearly identical clinical presentations, second episode was treated successfully with corticosteroid only. 

This is the first report of a primary autoimmune RE during postpartum period that is associated with ACA.  Immunologic causes should be considered early with any encephalitis. Given the risk of recurrence, relapse, and neurologic deterioration, regular monitoring is recommended, especially for female patients of child-bearing age.  Consistent with the current literature on autoimmune RE, steroid seems to be an effective treatment for ACA-associated RE.